History

Common presenting symptoms include double vision, weakness, unsteady gait, difficulty in swallowing, dysarthria, headache, drowsiness, nausea, and vomiting. Rarely, behavioral changes or seizures may be seen in children. Older children may have deterioration of handwriting and speech.

Pontine lesions usually present with any or all of the above signs and symptoms, depending on location and extension. Midbrain and lower brainstem/upper spinal cord signs and symptoms may be seen with extension of the neoplasm to involve these structures.

In infants and children presenting with failure to thrive, pontine glioma should be considered in the differential diagnosis.

Tectal lesions typically present with headache, nausea, and vomiting.

Hydrocephalus is a common presentation, especially for tumors in periaqueductal or fourth ventricle outflow locations, because these regions have less tolerance of growth and higher risk of obstructive hydrocephalus.

Tectal lesions may present with diplopia reflecting an internuclear ophthalmoplegia, indicating involvement of the medial longitudinal fasciculus. Parinaud syndrome also may be seen, with paralysis of upward gaze and accommodation, light-near dissociation (loss of pupillary reflex to light with preservation of pupilloconstriction in response to convergence), eyelid retraction, and convergence-retraction nystagmus.

Cervicomedullary lesions may present with sensory loss of the face (involvement of the trigeminal nucleus), dysphagia and/or dysphonia from lower cranial nerve involvement (commonly IX and X), long tract signs, and ataxia. Downbeating nystagmus and oculomyoclonus often are seen with medullary involvement.

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Causes

Although no familial tendency is prominent overall, an increased incidence of brainstem glioma has been observed consistently in patients with neurofibromatosis (up to 14% in some reports).

Thus far, no genetic or molecular markers have been recognized for brainstem gliomas.

In children irradiated for tinea capitis, an increased incidence of CNS tumors, especially meningiomas, gliomas, and nerve sheath tumors, has been reported. No specific reference is made in these reports to tumors of the brain stem. Radiotherapy-induced neoplasms tend to be more aggressive in their natural history than their de novo counterparts.